Clinical Features

Orbital varix is usually diagnosed between the ages of 10 to 30 years. However, it can be encountered in every age group, including neonates.49 This lesion generally involves the superior ophthalmic vein. However, other veins of the orbit can also be affected.

Most patients with an orbital varix develop positional proptosis. This is because the lesion has connections to the systemic venous circulation.6 The proptosis is exacerbated when the patient assumes a prone position, bends over, or performs a Valsalva maneuver.50 As the eye becomes more prominent, the palpebral fissure widens if the tumor is in the posterior orbit. Positional proptosis may not be seen in all cases of orbital varix because some small nondisten-sible varices have minimal or no connection to the systemic venous circulation (Figure 14.11).

Sometimes the lesion can have an acute onset with painful proptosis, compressive optic neuropathy, and decreased visual acuity, probably corresponding to the thrombosis or hemorrhage of the affected vein.51 Spontaneous orbital hemorrhage is more likely to occur with an orbital lymphangioma than with a varix.

FIGURE 14.11. (A) Facial photograph demonstrating the upper eyelid edema, subcon-junctival hemorrhage, and propto-sis in a 7-year-old girl who had undergone an orbitotomy for the orbital lesion. The lesion could not be identified during exploration. (B) Axial orbital CT with Valsalva maneuver showing the left orbital varix with ill-defined borders (C). Histopatho-logic examination of the lesion shows the dilated or ectatic vein with markedly thickened vessel walls. An organized recanalizing thrombus (arrow) is present within the lumen of the varix (VxL). (D| Facial photograph of the child 6 months after surgery shows no residual proptosis or eyelid abnormality.

Orbital varices have been divided into primary or secondary types. A primary orbital varix is confined to the orbit and is unassociated with an orbital or intracranial arteriovenous malformation. The secondary orbital varix develops in association with an intracra-nial arteriovenous malformation that shunts blood to the orbital venous system, causing the veins to dilate secondarily.23

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